酿酒酵母中人类CLN3或青少年神经元蜡样脂褐质沉积症基因的中断同源物：研究巴顿病的模型。
A disrupted homologue of the human CLN3 or juvenile neuronal ceroid lipofuscinosis gene in Saccharomyces cerevisiae: a model to study Batten disease.
作者信息
Guo W X, Mao C, Obeid L M, Boustany R M
机构信息
Department of Pediatrics, Duke University Medical Center, Durham, North Carolina 27710, USA.
出版信息
Cell Mol Neurobiol. 1999 Oct;19(5):671-80. doi: 10.1023/a:1006992704108.
Abstract
- In order to investigate the biological function of the human CLN3 gene that is defective in Batten disease, we created a yeast strain by PCR-targeted disruption of the yeast gene (YHC3), which is a homologue of the human CLN3 gene. 2. The phenotypic characterization revealed that the yhc3 delta mutants are more sensitive to combined heat and alkaline stress than the wild-type strains as determined by inhibition of cell proliferation. 3. This suggests that the yhc3 delta mutant is a good model to investigate the biological function of human CLN3 gene in mammalian cells and to understand the pathophysiology of juvenile Batten disease.
摘要
- 为了研究在巴滕病中存在缺陷的人类CLN3基因的生物学功能，我们通过PCR靶向破坏酵母基因（YHC3）创建了一种酵母菌株，该基因是人类CLN3基因的同源物。2. 表型特征表明，通过细胞增殖抑制测定，yhc3缺失突变体比野生型菌株对热和碱性联合应激更敏感。3. 这表明yhc3缺失突变体是研究人类CLN3基因在哺乳动物细胞中的生物学功能以及理解青少年巴滕病病理生理学的良好模型。
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本文引用的文献
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Mol Genet Metab. 1999 Apr;66(4):294-308. doi: 10.1006/mgme.1999.2834.
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An extracellular meiosis-promoting factor in Saccharomyces cerevisiae.酿酒酵母中的一种细胞外减数分裂促进因子。
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A yeast model for the study of Batten disease.用于研究巴滕病的酵母模型。
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Involvement of yeast sphingolipids in the heat stress response of Saccharomyces cerevisiae.酵母鞘脂参与酿酒酵母的热应激反应。
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BTN1, a yeast gene corresponding to the human gene responsible for Batten's disease, is not essential for viability, mitochondrial function, or degradation of mitochondrial ATP synthase.BTN1是一种与导致巴滕病的人类基因相对应的酵母基因，对于细胞活力、线粒体功能或线粒体ATP合酶的降解并非必需。
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